Table 1 Review of the reported ocular problems in Congenital Protein C deficiency
From: Ophthalmic manifestations of congenital protein C deficiency: a case report and mini review
Report | Reported cases | Involved eye | Anterior segment involvement | Posterior segment involvement | Global involvement |
|---|---|---|---|---|---|
Estelles A et al [1984] [6] | 1 | OU | Bilateral vitreous hemorrhage and intravitreal mass | ||
Marciniak E et al. [1985] [7] | 1 | OU | Corneal opacity Case1: Corneal opacity, pupil not visible in one eye Case2: Bilateral cataract, | Case 1: Vitreous opacity in one eye Case 2: total retinal detachment with retinal new and old hemorrhages | Microphthalmia |
Rappaport ES et al. [1987] [8] | 1 | OU | Hyperplastic vitreous bilaterally | ||
Pulido JS [1987] [9] | 1 | OU | Prominent iris vessels, shallow anterior chamber, synechia, cataract, retrolental membrane | Bilateral vitreal hemorrhage, and funnel-shaped retinal detachment in both eyes | |
Auletta MJ and Headington JT [1988] [10] | 1 | OU | Raised intraocular pressure, flattened anterior chambers, iris atrophy, lens adhesions | Bilateral retinal detachment | Bilateral leukocoria |
Hartman KR [1989] [11] | 1 | OU | Vitreal eye hemorrhages and | Intraparenchymal brain infarction | |
Hermsen VM et al [1990] [12] | 1 | OU | Vascularized lens, non-existent anterior chambers | Funnel-shaped retrolental mass, normal retina and optic nerve | Bilateral PHPV, microphthalmos |
Soria JM et al. [1985] [13] | 1 | Persistence of primary vitreous | Microphthalmia, irregular globe | ||
Cassels-Brown A et al. [1994] [14] | 2 | OU | Case 1: Posterior embryotoxon, shallow anterior chambers, ectropion uvea, posterior synechia Case 2: marked periorbital edema and hemorrhagic conjunctival chemosis | Case 1: subconjunctival hemorrhage, a right sided retinal arterial occlusion and bilateral florid retinal hemorrhages with swollen hemorrhagic optic discs secondary to retinal venous occlusions, vitreous hemorrhages retinal detachment in one eye Case 2: hazy media, minor bilateral vitreous hemorrhages, and minor right sided retinal hemorrhage | Case 1: Strabismus, nystagmus, leukocoria Case 2: early birth 25 wk., end up to death at 23 days of life because of some subarachnoid hemorrhage and with worsening lung function |
Acheson JF et al. [1994] [15] | 4 | OD | Bilateral recurrent ischemic optic neuropathy | Association with Protein S deficiency, antithrombin III | |
Dreyfus M et al. [1995] [16] | 9 | OU | Unilateral or bilateral blindness 6/9 cases bilateral PHPV | ||
Hattenbach LO et al. [1999] [5] | 2 | OU | Case 1. Shallow anterior chamber Case 2. Shallow anterior chamber, posterior synechia | Case 1. Left retrolental opacities, funnel retinal detachment, vitreous hemorrhage Case 2. Right vitreous hemorrhage, funnel retinal detachment | Case 1. Strabismus, microphthalmos Case 2. Microphthalmos |
Ergenekon E et al. [2000] [17] | 1 | OS | Eye ultrasound revealed an10x7 mm hyperechogenic structure underneath. Retina of the left eye consistent with subretinal hemorrhage | Leukocoria | |
Churchill AJ et al. [2001] [18] | 2 | OU | Case2. Right leukocoria | Case 1. Bilateral central retinal vein occlusions, vitreous hemorrhage, and a right central retinal artery occlusion Case 2. Total retinal detachment and a left macular hemorrhage | |
Paysse EA [2002] [19] | 1 | OU | Flat anterior chamber | Bilateral retinal detachment, fibrotic hyaloid arteries | |
Sirachainan N et al [2003] [20] | 2 | OU | Preserving eye function, high myopia, cerebral palsy | ||
Park UC et al. [2005] [21] | 1 | OU | Bilateral corneal opacity, microphthalmia, posterior synechia, pupillary membrane, shallow anterior chamber | Vitreoretinopathy suggesting, PHPV, intravitreal masses, funnel-shaped retinal detachment with bilateral retinal dysplasia | Bilateral leukocoria, no microphthalmos, blind OU |
de Lemus-Varela ML [2005) [22] | 1 | OU | Vitreous hemorrhage | ||
Douglas AG et al. [2010] [23] | 1 | OU | No red reflex OU | Vitreoretinal dysplasia, severe hazy media in one eye and OU PFV | Leukocoria OS, bilateral microphthalmia,, deranged VEP, poor vision, nystagmus |
Wang BZ [2012] [24] | 1 | OU | Macular hypoplasia with blond retinal background | Doll’s eye movements, no fix and follow movement of the eyes, ponto-cerebellar hypoplasia, nystagmus, strabismus, Flat ERG | |
Desai S et.al. [2014] [25] | 1 | OU | Combined central retinal venous and arterial obstruction | Severe Type II protein C deficiency with factor V Leiden mutation, Glaucoma | |
Almarzouki HS et al [2016] [26] | 2 | OU | Peter’s anomaly | ||
Baothman AA et al. [2017] [27] | 5 | OU | Peter’s anomaly, corneal opacity (3/5) | Blindness (4/5OU), ocular hemorrhage shortly after birth resulted in visual loss (1/5) | |
Present study | 1 | OU | Left eye shallow anterior chamber, cataract, leukoma, posterior synechia | Dysplastic retina and total retinal detachment in the other eye | Flat ERG, fluorescent spots |